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Vol 8, No 1
Vol 8, No 1 February 2012 ISSN 1708-8569
 
Commentary
Review articles
Original articles
Brief reports
Case reports
Clinical imaging
Correpondence
   
Commentary:
Cross-cultural application of Achenbach System of Empirically Based Assessment: instrument translation in Chinese, challenges, and future directions
  Jianghong Liu, Patrick Leung, Ruin Sun, Hong-Tian Li, Jian-Meng Liu
  Philadelphia, USA

Author Affiliations: School of Nursing, University of Pennsylvania, USA (Liu J); Department of Psychology, The Chinese University of Hong Kong, Hong Kong, China (Leung P); Department of Psychology, Nanjing Normal University, China (Sun R); Peking University Health Science Center, China (Li HT, Liu JM)

Corresponding Author: Jianghong Liu, PhD, University of Pennsylvania School of Nursing and Medicine, 418 Curie Blvd., Room 426, Claire M. Fagin Hall, Philadelphia, Pennsylvania 19104-6096, USA (Tel: (215) 898-8293; Email: jhliu@nursing.upenn.edu)

doi: 10.1007/s12519-011-0329-x

  [Abstract] [Full Text] [PDF]  
Review articles:
Child mortality in India: a complex situation
  Rohini Ghosh
   

Author Affiliations: Epidemiologist (Senior Research Fellow), South Asia Network for Chronic Disease, Public Health Foundation of India (Ghosh R)

Corresponding Author: Rohini Ghosh, C-1/52, Safdarjung Development Area, First Floor, New Delhi 110 016, India (Tel: 011-26513082, 011-26513099 ext 126; Email: rohini.ghosh@gmail.com; rohini.ghosh@phfi.org)

doi: 10.1007/s12519-012-0331-y

Background: The countdown database to track the maternal and child survival rate, as set by the Millennium Development Goal, reported recently that India's progress is not satisfactory in reducing newborn and child deaths.

Data sources: Articles on neonatal and child mortality in India were accessed from PubMed/MEDLINE. Risk factors associated with neonatal and child mortality were reviewed in three crucial phases of pregnancy, childbirth and postnatal period.

Results: The review revealed economic disparity acts through various avenues of cultural belief and restrictions and is indirectly associated with care seeking behavior and utilization of health care, resulting in slow decline of child mortality rate in India. Secondly, cultural norms, practices, and beliefs are strongly associated with high neonatal mortality, contributing to the sluggish decline of overall child survival rate. Proximate determinants of child mortality, i.e., income, cultural behavior and beliefs, in multiplicity of Indian cultures, are closely associated with health seeking behavior, antenatal care, delivery practices and postnatal care of infants.

Conclusions: Apart from raising awareness among community leaders, family members responsible for care giving should be specially targeted for removal of hostile perceptions and barriers for improvement of child survival. Also there is need for developing new strategies for health education based on indigenous concerns, addressing socio-cultural barriers.

Key words: child mortality; economic disparity; neonatal death; stagnation

                     World J Pediatr 2012;8(1):11-18

  [Abstract] [Full Text] [PDF]  
Diagnosis and management of cow's milk protein allergy in infants
  Elisabeth De Greef, Bruno Hauser, Thierry Devreker, Gigi Veereman-Wauters, Yvan Vandenplas
   

Author Affiliations: Department of Pediatrics, UZ Brussel, Brussels, Belgium (De Greef E, Hauser B, Devreker T, Veereman-Wauters G, Vandenplas Y)

Corresponding Author: Yvan Vandenplas, MD, PhD, Department of Pediatrics, UZ Brussel, Vrije Universiteit Brussel, Brussels, Belgium (Tel: 02 477 57 81; Fax: 02 477 57 83; Email: yvan.vandenplas@uzbrussel.be)

doi: 10.1007/s12519-012-0332-x

Background: Cow's milk protein allergy (CMPA) is frequently suspected in infants with a variety of symptoms. A thorough history and careful clinical examination are necessary to exclude other underlying diseases and to evaluate the severity of the suspected allergy. Care should be taken to diagnose CMPA adequately to avoid an unnecessary diet.

Data sources: We make recommendations based on systematic literature searches using the best-available evidence from PubMed, Cumulative Index to Nursing and Allied Health Literature, and bibliographies.

Results: Skin prick tests, patch tests and serum specific IgE are only indicative of CMPA. Breastfed infants have a decreased risk of developing CMPA; an elimination diet for the mother is indicated if CMPA is confirmed. If a food challenge is positive in formula fed infants, an extensively hydrolysed formula and cow's milk-free diet is recommended. If symptoms do not improve, an amino acid based formula should be considered. In severe CMPA with life-threatening symptoms, an amino-acid formula is recommended.

Conclusions: Elimination diet by a double-blind placebo controlled food challenge is the gold standard for diagnosis. Elimination of the offending allergen from the infants' diet is the main treatment principle.

Key words: amino acid formula; cow's milk protein allergy; extensive hydrolysate; partial hydrolysate; soy formula

                  World J Pediatr 2012;8(1):19-24

  [Abstract] [Full Text] [PDF]  
Understanding Bartter syndrome and Gitelman syndrome
  Oliver T. Fremont, James C.M. Chan
   

Author Affiliations: Tufts University School of Medicine; The Barbara Bush Children's Hospital, Maine Medical Center, Portland, Maine, USA (Fremont OT, Chan JCM)

Corresponding Author: James C. M. Chan, M.D., Professor of Pediatrics, Tufts University School of Medicine; Director of Research, The Barbara Bush Children's Hospital, Maine Medical Center, 22 Bramhall Street, Portland, Maine 04102-3175, USA (Tel: 207-662-2439; Fax: 207-662-6272; Email: cmchanj@comcast.net)

doi: 10.1007/s12519-012-0333-9

Background: We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome.

Data sources: Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011.

Results: The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition.

Conclusions: Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with sypmtoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

Key words: Bartter syndrome; blood pressure control; Gitelman syndrome; NCCT; NKCC2; potassium; sodium 

World J Pediatr 2012;8(1):25-30

  [Abstract] [Full Text] [PDF]  
Original articles:
Impact of childhood obesity treatment on body composition and metabolic profile
  Marja Kalavainen, Pauliina Utriainen, Esko Vanninen, Matti Korppi, Outi Nuutinen
   

Author Affiliations: Departments of Pediatrics and Clinical Nutrition, Kuopio University Hospital, Kuopio, Finland (Kalavainen M); Department of Pediatrics, Kuopio University Hospital and University of Eastern Finland, Finland (Utriainen P); Department of Clinical Physiology and Nuclear Medicine, Kuopio University Hospital and University of Eastern Finland, Finland (Vanninen E); Pediatric Research Center, Tampere University and University Hospital, Tampere, Finland (Korppi M); Institute of Public Health and Clinical Nutrition, Department of Clinical Nutrition, University of Eastern Finland, Kuopio, Finland (Nuutinen O)

Corresponding Author: Matti Korppi, Pediatric Research Center, Tampere University and University Hospital, FinMed-3 building, FIN-33014 Tampere University, Finland (Tel: +358-3-35518407; Fax: +358-3-35538420; Email: matti.korppi@uta.fi)

Background: Childhood obesity is associated with adverse changes in cardiometabolic risk factors. A family-oriented group program stressing a health-promoting lifestyle has been more effective than routine counselling in the treatment of obesity in school children. The aim of the present study was to compare the impact of group program and routine councelling on body composition and metabolic profile, and to evaluate the associations of changes in adiposity with levels of cardiometabolic risk factors.

Methods: Seventy obese prepubertal children were randomized into family-oriented group program (15 sessions for parents and children) and routine counselling (2 appointments for children). Body mass index (BMI), body composition and different metabolic risk factors were assessed before and after the 6-month intervention.

Results: Waist/height decreased more in the children attending the group treatment, but there were no significant differences between treatment arms in the changes of metabolic risk factors. When the arms were analyzed as combined, serum triglycerides decreased significantly if BMI standard deviation score (BMI-SDS) decreased ≥0.5. Serum fasting insulin decreased if BMI-SDS decreased ≥0.25.

Conclusions: Obesity-related metabolic risk factors reduced in prepubertal children if BMI-SDS decreased substantially. This result was not dependent on which intervention, family-oriented group program or routine counselling, was used.

Key words: body composition; childhood obesity; metabolism

World J Pediatr 2012;8(1):31-37

  [Abstract] [Full Text] [PDF]  
Cross-cultural adaptation and reliability of pediatric sleep questionnaire in assessment of sleep-disordered breathing in the Malay speaking population
  AL Hasniah, AR Jamalludin, AW Norrashidah, MZ Norzila, K Asiah, A Rus Anida, A Ahmad Fadzil, Z Ramli, H Samsinah
   

Author Affiliations: Pediatric Department, Universiti Kebangsaan Malaysia Medical Centre, Malaysia (Hasniah AL); Department of Community Health, International Islamic University Malaysia (Jamalludin AR); Pediatric Department, Serdang Hospital, Malaysia (Norrashidah AW); Pediatric Institute, Kuala Lumpur Hospital, Malaysia (Norzila MZ, Asiah K); Pediatric Department, Penang Hospital, Malaysia (Rus Anida A); Pediatric Department, Tengku Ampuan Afzan Hospital, Malaysia (Ahmad Fadzil A); Institute for Health Systems Research, Malaysia (Ramli Z); University of Malaya, Kuala Lumpur, Malaysia (Samsinah H)

Corresponding Author: Assoc. Prof. Dr Abdul Latif Hasniah, Pediatric Department, Universiti Kebangsaan Malaysia Medical Centre, Jln Yaacob Latif, 56000 Cheras, Kuala Lumpur, Malaysia (Tel: +6 03 91455395/91455380; Fax: +603-91456637; Email: hasniah@ppukm.ukm.my)

 

doi: 10.1007/s12519-011-0279-3

Background: Sleep-disordered breathing (SDB) is common but often underdiagnosed in children. The Pediatric Sleep Questionnaire developed by University of Michigan, USA (English UM PSQ) has high sensitivity and specificity in identifying children with sleep-disordered breathing. This study aimed to translate and adapt the English UM PSQ into Malay language as a screening tool to assess SDB among the Malay speaking population. The second objective was to determine the psychometric measurements of the translated UM PSQ (Malay UM PSQ).

Methods: The Malay UM PSQ was translated through forward-backward translation techniques by two independent accredited bodies and reviewed by a panel of experts. The questionnaire was tested in two phases. The respondents were from hospital staffs with children and parents of primary school children aged 6-10 years. The reliability of questionnaires was measured by Cronbach's α and Kappa (κ) statistics.

Results: The overall scale of internal consistency of the Malay UM PSQ was good, i.e., Cronbach's α = 0.760 (α = 0.457, 0.608 and 0.688 for snoring, sleepiness and behavioral domains respectively). The English UM PSQ also had good internal consistency at α = 0.753 (α = 0.589, 0.524, to 0.793 for snoring, sleepiness and behavioral domains respectively). Test-retest reliability for most items was good with correctness of >85.0% in all items. Only one item was seen in the Malay UM PSQ with κ=0.348, while the remaining ranged from κ=0.489 to 0.811. For the English UM PSQ, κ ranged from 0.660 to 0.945.

Conclusion: Both English and Malay UM PSQ have acceptable psychometric measurement properties as screening tools to assess SDB in the Malay speaking population.

Key words: pediatric; questionnaire; reliability; sleep-disordered breathing

                                       World J Pediatr 2012;8(1):38-42

  [Abstract] [Full Text] [PDF]  
Epidemiological features and risk factor analysis of children with acute lung injury
  Yan Li, Quan Wang, Hui Chen, Heng-Miao Gao, Tong Zhou, Su-Yun Qian
   

Author Affiliations: Department of PICU, Beijing Children's Hospital of Capital Medical University, No. 56 Nan Li Shi Road, Beijing 100045, China (Li Y, Wang Q, Chen H, Gao HM, Qian SY); Department of Nuclear Medicine, Dongzhimen Hospital affiliated to Beijing University of Traditional Chinese Medicine, Beijing 100700, China (Zhou T)

Corresponding Author: Su-Yun Qian, MD, Department of PICU, Beijing Children's Hospital of Capital Medical University, No. 56 Nan Li Shi Road, Beijing 100045, China (Tel: +86-010- 59612765; Fax: +86-010-59718726; Email: syqian@yahoo.com.cn)

doi: 10.1007/s12519-012-0334-8

Background: Acute lung injury (ALI)/acute respiratory distress syndrome (ARDS) represents a devastating complication observed in critical care medicine. The purpose of this study is to investigate the epidemiological aspects of ALI/ARDS in pediatric intensive care unit (PICU) and risk factors of mortality.

Methods: Patients with ALI/ARDS in PICU of Beijing Children's Hospital, a tertiary medical center from November 1, 2005 to October 31, 2006 were included in this prospective study. We identified the risk factors for underlying diseases and mortality during a 3-month follow-up using multivariate logistic regression analysis.

Results: In 562 critically ill patients admitted to PICU of Beijing Children's Hospital, there were 15 ALI-non ARDS patients and 29 ARDS patients, resulting in an incidence of 7.8% (44/562). The mortality rate of ARDS was 24.1% (7/29) and that of ALI/ARDS was 18.2% (8/44). At a 3-month follow-up, 12 patients died after being discharged from PICU and the total mortality rate was 45.5% (20/44). ALI/ARDS patients with pulmonary disease had better outcomes than those with extra-pulmonary involvements (P<0.05). Discharge against medical advice, low PaO2/FiO2 during hospital stay and high PaCO2 on PICU admission were risk factors of mortality.

Conclusions: ARDS has a high mortality rate in PICU, especially in those with extra-pulmonary diseases. In addition to aggressive medical management of comorbidity, lung protection and avoidance of discharge against medical advice will decrease the mortality.

Key words: acute lung injury; acute respiratory distress syndrome; children; epidemiology; risk factor

                    World J Pediatr 2012;8(1):43-46

  [Abstract] [Full Text] [PDF]  
The course of early-onset multiple sclerosis in Iraqi children
  Hasan A. Al-Hamadani, Atheer S. Abdalla, Atheer J. Al-Saffar
   

Background: Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system that is increasingly recognized worldwide in children and adolescents. The current study aimed at identifying the clinical characteristics of MS with onset under 18 years of age.

Methods: This cross-sectional study was conducted in the Multiple Sclerosis Center archive system in Baghdad Teaching Hospital during the period from March 1 to May 15, 2008. The records of 1125 MS patients from 2000 to 2008 were reviewed. Among them 77 patients had the onset of MS under 18 years of age.

Results: Two thirds of the patients were female (a female/male ratio of 1.6:1). The mean age of the patients at the onset of the disease was 14.95±3.21 years, and the mean time between the first and second attacks was 3.06±4.09 years. Seventy patients (90.9%) had an initial course of relapse remitting MS. Among them 9 (12.9%) progressed to secondary progressive MS after a mean duration of 9.87±4.14 years. The remaining 7 patients had primary progressive MS associated with optic neuritis  and brain stem lesion. Fifty-nine (76.6%)  patients had monofocal signs and 18 (23.4%) had polyfocal signs. The mean extended disability status scale score was 4.15±2.17 and the mean progression index was 1.44±2.31. There was a strong inversed correlation between the progression index and interval between the first and second attacks (P=0.0001).

Conclusions: The results of the present study show that the course of MS in Iraqi children and adolescents is more aggressive than in children from other countries. This finding needs to be evaluated by further studies.

Key words: clinical presentation; early-onset; multiple sclerosis

                    World J Pediatr 2012;8(1):47-51

  [Abstract] [Full Text] [PDF]  
Acute phase 99mTc-dimercaptosuccinic acid scan in infants with first episode of febrile urinary tract infection
  Nikoleta Printza, Evagelia Farmaki, Kalliopi Piretzi, George Arsos, Konstantinos Kollios, Fotios Papachristou
   

Author Affiliations: 1st Pediatric Department, Aristotle University of Thessaloniki, Hippokration General Hospital, Greece (Printza N, Farmaki E, Piretzi K, Papachristou F); Department of Nuclear Medicine, Hippokration General Hospital, Greece (Arsos G); 3rd Pediatric Department, Aristotle University of Thessaloniki, Hippokration General Hospital, Greece (Kollios K)

Corresponding Author: Nikoleta Printza, MD, 1st Pediatric Department, Aristotle University, Hippokration Hospital, 49 Konstantinoupoleos St., GR-546 42 Thessaloniki, Greece (Tel: +30-2310-892425; Fax: +30-2310-865189; Email: nprintza@in.gr)

 

doi: 10.1007/s12519-012-0335-7

Background: 99mTc-dimercaptosuccinic acid (DMSA) scan is the golden standard for the diagnosis of acute pyelonephritis and renal scaring. We investigated the use of acute phase DMSA scan in infants presented promptly to the hospital because of the first episode of their febrile urinary tract infection (UTI).

Methods: Ninety-eight infants with microbiologically confirmed first episode of febrile UTI were studied. DMSA scans were carried out within 7 days in these infants after admission. Infants with an abnormal acute DMSA scan underwent a second DMSA scan 6-12 months later.

Results: Overall, acute DMSA scan was abnormal in 16 (16.3%) of the 98 patients. There were no differences in sex, age, fever over 38.5°C, blood inflammation indices, or evidence of vesicoureteral reflux (VUR) between patients with normal and abnormal acute DMSA scan (P>0.05). However, infants with grade III to V VUR as well as those with delayed treatment presented significantly increased renal involvement by acute DMSA scan (P<0.05). The sensitivity and specificity of abnormal acute DMSA scan to predict grade III to V VUR were 50% and 88% respectively. Its positive and negative likelihood ratios were 4.16 and 0.57, respectively. Of 16 children with abnormal initial DMSA scan results, 14 underwent a second DMSA scan. Follow-up DMSA scans were normal in 12 of the 14 children.

Conclusions: Parenchymal damage found in a minority of infants with febrile UTI presented promptly to the hospital. Acute phase DMSA scan should be carried out only in selected patients. An abnormal acute DMSA scan is a moderate predictor for dilated VUR and its ability to exclude VUR is restricted.

Key words: 99mTc-dimercaptosuccinic acid; infants; pyelonephritis; vesicoureteral reflux;  urinary tract infection

World J Pediatr 2012;8(1):52-56

  [Abstract] [Full Text] [PDF]  
Laparoscopic pyloromyotomy: is a knife really necessary?
  Vishesh Jain, Subhasis Roy Choudhury, Rajiv Chadha, Archana Puri,
   

Author Affiliations: Department of Pediatric Surgery, Lady Hardinge Medical College, Kalawati Saran Children's Hospital, New Delhi-110001, India (Jain V, Roy Choudhury S, Chadha R, Puri A, Naga AS)

Corresponding Author: Vishesh Jain, D-9, Green Park extension, New Delhi, India-110016 (Tel: 91-011-32531393; Email: dr.vishesh79@gmail.com) This work was presented at the 22nd Congress of the Asian Association of Pediatric Surgeons held on February 22-24, 2010, Kaula Lampur, Malaysia.

doi: 10.1007/s12519-011-0278-4

Background: Laparoscopic pyloromyotomy (LP) is currently accepted as a suitable treatment modality for infantile hypertrophic pyloric stenosis (IHPS). In this report, we describe some technical modifications of LP using a 3- or 5-mm hook with electrocautery as a substitute for a knife for incising the pylorus. The outcomes of LP using a standard retractable pyloromyotomy knife are compared with those of LP using a hook electrocautery.

Methods: The patients with ultrasound proven IHPS who had undergone LP in a single institution from December 2008 to April 2010 were retrospectively analyzed. Incision on the pylorus was made with a 3-mm pyloromyotomy knife in the initial 12 cases. However, in the latter part of the study, a 3- or 5-mm hook with electrocautery was used for the incision. A Maryland dissector was used for completing the pyloromyotomy. The results were compared in terms of duration of surgery, complications, time taken to establish the first full feed, requirement of analgesics, postoperative emesis, and postoperative stay in the hospital. Independent sample t test and the Chi-square test were used for statistical analysis.

Results: Of the 27 patients analyzed, 12 underwent LP using a pyloromyotomy knife and the remaining 15 patients were operated on using a hook with electrocautery instead of the knife. The operating time, time taken to establish the first full feed, and duration of hospital stay were comparable among the two groups with no statistically significant difference. No complications were recorded in either group.

Conclusions: Use of hook electrocautery for incising the pylorus provides a bloodless field without affecting the postoperative recovery and outcome. It also obviates any need of specialized instruments like a pyloromyotomy knife or other sharp instruments for pyloric incision.

Key words: infantile hypertrophic pyloric stenosis; laparoscopy; pyloromyotomy                   

World J Pediatr 2012;8(1):57-60
  [Abstract] [Full Text] [PDF]  
Niemann-Pick disease type C: analysis of 7 patients
  Hui Xiong, Xin-Hua Bao, Yue-Hua Zhang, You-Ning Xu, Jiong Qin, Hui-Ping Shi, Xi-Ru Wu
   

Author Affiliations: Department of Pediatrics, Peking University First Hospital, Beijing 100034, China (Xiong H, Bao XH, Zhang YH, Qin J, Wu XR); Department of Pediatrics, Beijing Changping District Hospital, Beijing, China (Xu YN); Department of Genetics, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China (Shi HP)

Corresponding Author: Xi-Ru Wu, MD, Department of Pediatrics, Peking University First Hospital, Beijing 100034, China (Tel: 0086-10-83573238; Fax: 0086-10-66530532; Email: xh_bjbj@yahoo.com.cn)

doi: 10.1007/s12519-011-0284-6

Background: Niemann-Pick disease type C (NP-C), derived from mutation of the NPC1 or NPC2 gene, is one of the recessive lysosomal lipid storage disorders that are difficult to diagnose and treat. Since NP-C has been rarely reported in China, we reviewed 7 patients with NP-C.

Methods: The 7 patients had been diagnosed with NP-C from 2007 to 2010 at our department and their laboratory and clinical data were analyzed.

Results: The 7 patients, 5 males and 2 females, included 4 patients of late infantile subtype and 3 patients of juvenile subtype, in which patients 2 and 3 were siblings. Their clinical symptoms occurred from 4 to 10 years of age, exhibiting as progressive cognitive and language impairment as well as motor retrogression. Six patients were caught by focal or generalized seizures from 1 to 4 years after the onset of the disease. Vertical supranuclear gaze palsy, dysarthria, dysphagia, internal rotation and adduction of bilateral hands and splenomegaly occurred following the progress of clinical symptoms. Five patients had laughter-cataplexy. MRI showed mild brain atrophy in 6 patients. Reduction of total cholesterol, high density lipoprotein cholesterol and low density lipoprotein cholesterol occurred in 6 patients. Sea-blue cells and Niemann-Pick cells were found in bone marrow smears. The activity of acid sphingomyelin enzyme was normal or only slightly lower. Supporting or symptomatic treatment improved common clinical symptoms.

Conclusions: NP-C is a rare autosomal recessive inherited lysosomal storage disease that affects the intellectual development of children and may lead to dementia, vegetative state or death. Clinical features of this disease include vertical supranuclear gaze palsy, seizures and cataplexy. Laboratory features include abnormal plasma cholesterol level, and sea-blue cells and Niemann-Pick cells in bone marrow smears. The treatments of the disease include supporting or symptomatic administration.

Key words: Niemann-Pick disease type C; sea-blue cells; vertical supranuclear gaze palsy

World J Pediatr 2012;8(1):61-66

  [Abstract] [Full Text] [PDF]  
A novel heterozygous NR1H4 termination codon mutation in idiopathic infantile cholestasis
  Xiu-Qi Chen, Lin-Lin Wang, Qing-Wen Shan, Qing Tang, Ya-Nan Deng, Shu-Jun Lian, Xiang Yun
   

Author Affiliations: Department of Pediatrics, First Affiliated Hospital, Guangxi Medical University, Nanning, 530021 Guangxi, China (Chen XQ, Wang LL, Shan QW, Tang Q, Deng YN, Lian SJ, Yun X)

Corresponding Author: Lin-Lin Wang, Department of Pediatrics, First Affiliated Hospital, Guangxi Medical University, Nanning, 530021 Guangxi, China (Tel: +86-771-5356505; Fax: +86-771-5356781; Email: WLL276@yahoo.com.cn)

doi: 10.1007/s12519-011-0299-z

Background: This study aimed to evaluate the genetic effect of the NR1H4 gene in the pathogenesis of idiopathic infantile cholestasis of Chinese subjects in Guangxi, China.

Methods: Seventy-eight patients with idiopathic infantile cholestasis served as a study group and 95 infants without cholestasis as controls. Genomic DNA was extracted from peripheral venous blood leucocytes by phenol chloroform procedures. Polymerase chain reaction (PCR) was used to amplify all coded exons of NR1H4, and single-strand conformation polymorphism (SSCP) was used to analyze all amplification fragments. The PCR products with abnormal bands in SSCP were sequenced using an ABI 3100 sequencer.

Results: A novel heterozygous termination codon mutation in NR1H4 exon 5 (NR1H4 R176X, CGA-TGA) was found in one of the 78 patients. The patient with mutation R176X had high levels of bilirubin, alanine aminotransferase, γ-glutamyltransferase, cirrhosis and ascites despite biliary tract flushing procedures and drug therapy. In the other patients and controls, no mutation was detected.

Conclusions: Heterozygous termination codon mutation of NR1H4 R176X was found in idiopathic infantile cholestasis. The novel mutation is useful to establish particular characteristics for differential diagnosis of idiopathic infantile cholestasis and to determine the influence of such gene defects in the prognosis.

Key words: cholestasis; farnesoid X receptor; infant; mutation; NR1H4

                    World J Pediatr 2012;8(1):67-71

  [Abstract] [Full Text] [PDF]  
Brief reports:
Evaluation of adhesion molecules CD64, CD11b and CD62L in neutrophils and monocytes of peripheral blood for early diagnosis of neonatal infection
  Ferah Genel, Fusun Atlihan, Nesrin Gulez, Elif Kazanci, Canan Vergin, Demet Tumay Terek, Ozlem Cengel Yurdun
   

Author Affiliations: Department of Pediatrics, Dr Behçet Uz Children's Hospital, Alsancak, Izmir, Turkey (Genel F, Atlihan F, Gulez N, Kazanci E, Vergin C, Tumay Terek D, Cengel Yurdun O)

Corresponding Author: Ferah Genel, MD, Department of Pediatrics, Dr Behçet Uz Children's Hospital, 1374 sokak, No: 11, 35210, Alsancak, Izmir, Turkey (Tel: +90 232 489 56 56; Fax: +90 232 489 23 15; Email: ferahgen@yahoo.com)

doi: 10.1007/s12519-011-0304-6

Background: This study was undertaken to assess the value of neutrophils CD11b, CD64, and CD62L for the early diagnosis of neonatal infection.

Methods: Eighty-four neonates who were followed up for a suspected neonatal infection were included in this study. They were assigned into an infection group (n=49) and a non-infection group (n=35). Healthy neonates served as controls (n=35). A full sepsis screening was performed and neutrophil and monocyte expressions of CD11b, CD64 and CD62L were determined by flow cytometry.

Results: The expressions of CD64 and CD11b were significantly enhanced in the infection group compared to the non-infective group and the controls.

Conclusions: CD64 expression on neutrophils and monocytes is a useful diagnostic marker for the early diagnosis of neonatal infection. Combination of CD64, CD11b and C reactive protein further enhances the sensitivity of the expression and its negative predictive value.

Key words: adhesion molecule; markers; neonatal infection

                   World J Pediatr 2012;8(1):72-75

  [Abstract] [Full Text] [PDF]  
Maternal obesity associated with inflammation in their children
  Karen L. Leibowitz, Reneé H. Moore, Rexford S. Ahima, Albert J. Stunkard, Virginia A. Stallings, Robert I. Berkowitz, Jesse L. Chittams, Myles S. Faith, Nicolas Stettler
   

Author Affiliations: The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA (Leibowitz KL, Stallings VA, Berkowitz RI, Faith MS, Stettler N); UMDNJ-Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA (Leibowitz KL); University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA (Moore RH, Ahima RS, Stunkard AJ, Stallings VA, Berkowitz RI, Chittams JL, Faith MS, Stettler N)

Corresponding Author: Karen L. Leibowitz, MD, Department of Pediatrics, Division of Gastroenterology and Nutrition, UMDNJ-RWJMS, 89 French Street, 2nd Floor, New Brunswick, NJ 08901, USA (Tel: 732-235-6109; Fax: 732-235-6381; Email: leibowka@umdnj.edu)

doi: 10.1007/s12519-011-0292-6

Background: This study explored the association between maternal obesity during pregnancy and the inflammatory markers, tumor necrosis factor-α, interleukin-6 and high sensitivity C-reactive protein (hs-CRP), and the cytokine, adiponectin, in the offspring.

Methods: Weight, height, Tanner stage and biomarkers were measured in thirty-four 12-year-old children, from the Infant Growth Study, who were divided into high risk (HR) and low risk (LR) groups based on maternal pre-pregnancy body mass index (BMI).

Results: The two groups differed markedly in their hs-CRP levels, but no group difference was found for the other three biomarkers. The odds ratio (OR) of HR children having detectable hs-CRP levels was 16 times greater than that of LR children after adjusting for confounding variables, including BMI z-score, Tanner stages and gender (OR: 16; 95% CI: 2-123).

Conclusions: These results suggest that maternal obesity during pregnancy is associated with later development of elevated hs-CRP in the offspring, even after controlling for weight.

Key words:  children; hs-C-reactive protein; inflammation; maternal obesity

World J Pediatr 2012;8(1):76-79

  [Abstract] [Full Text] [PDF]  
Case reports:
Cytomegalovirus enterocolitis presenting as abdominal compartment syndrome in a premature neonate
  Steven L. Lee, Hege Johnsen, Harry Applebaum
   

Author Affiliations: Division of Pediatric Surgery, Harbor-UCLA, Torrance, California, USA (Lee S); Department of General Surgery (Johnsen H) and Division of Pediatric Surgery (Applebaum H), Kaiser Permanente, Los Angeles Medical Center, Los Angeles, California, USA

Corresponding Author: Steven L. Lee, MD, Division of Pediatric Surgery, Harbor-UCLA Medical Center, Torrance, CA 90509, USA (Tel: (310) 222-2706; Fax: (310) 782-1562; Email: slleemd@yahoo.com)

Background: Cytomegalovirus (CMV) enterocolitis is an uncommon intestinal disorder of newborns that is often initially misdiagnosed as necrotizing enterocolitis.

Methods: We treated a premature twin boy with CMV enterocolitis who presented with abdominal compartment syndrome requiring urgent decompression. All patients with neonatal CMV enterocolitis reported were reviewed.

Results: Nine previously reported patients with neonatal CMV enterocolitis presented with abdominal distention and signs of sepsis. At the time of surgery, either perforation or stricture was identified. The current report is the first to present with clinical signs of abdominal compartment syndrome.

Conclusion: CMV is a rare cause of neonatal enterocolitis. Surgical intervention is required for bowel perforation, stricture, or abdominal compartment syndrome.

Key words: abdominal compartment syndrome; cytomegalovirus; enterocolitis; necrotizing enterocolitis

                   World J Pediatr 2012;8(1):80-82

  [Abstract] [Full Text] [PDF]  
Hemangioendothelioma of the right atrial appendage associated with pericardial effusion in an infant
  Yu-Jia Wang, Ze-Wei Zhang, Jin Yu, Hong-Feng Tang, Zhan Gao, Fang-Qi Gong
   

Author Affiliations: Department of Cardiology (Wang YJ, Gong FQ), Department of Cardiothoracic Surgery (Zhang ZW, Gao Z), Department of Ultrasonography (Yu J), and Department of Pathology (Tang HF), Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China

Corresponding Author: Fang-Qi Gong, MD, Department of Cardiology, Children's Hospital, Zhejiang University School of Medicine, 57 Zhugan Xiang, Hangzhou 310003, China (Tel: +86-571-87061007; Fax: +86-571-87033296; Email: gongfangqi@zju.edu.cn)

doi: 10.1007/s12519-012-0345-5

Background: Cardiac hemangioma is a rare cause for pericardial effusion. We present a case of hemangioendothelioma of the right atrial appendage associated with pericardial effusion in an infant.

Methods: The patient was a 49-day-old infant transferred to our department because of the presence of pericardial effusion. Two-dimensional transthoracic echocardiography revealed moderate pericardial effusion and a 24 mm × 16 mm hypoechoic mass located on the right side of the right ventricular outflow tract and in front of the aorta.

Results: The infant underwent an exploratory median sternotomy. In the pericardial cavity, 120mL transudate was observed. A 15 mm × 15 mm encapsulated mass of soft tissue was located in the pericardial cavity and involved the right atrial appendage. The tumor with right atrial appendage was completely removed. Pathological examination revealed wide sessile implant basis of the tumor into the the myocardium of the right atrial appendage, with no affection to the endocardium. Hemangioendothelioma was confirmed histopathologically. Echocardiographic examination 2 years after operation revealed that the infant was free from tumor recurrence.

Conclusions: Pericardial effusion may be caused by hemangioma of the right atrial appendage. The diagnosis of cardiac hemangioma is based on imaging examination and histopathological studies.

Key words: cardiac hemangioma; infant; pericardial effusion; right atrial appendage

                    World J Pediatr 2012;8(1):83-85

  [Abstract] [Full Text] [PDF]  
Clinical imaging:
Through the looking-glass what you found there
  Giampiero Soccorso, George K Ninan
 
  [Abstract] [Full Text] [PDF]  
Correpondence:
Correspondence
  Nejat AKAR, et al
 
  [Abstract] [Full Text] [PDF]  
   
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